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C1-inhibitor (C1-inh, C1 esterase inhibitor) is a protease inhibitor belonging to the serpin superfamily. [5] Its main function is the inhibition of the complement system (C1r, C1s) to prevent spontaneous activation but also as the major regulator of the contact system (PK, FXIIa, and FXIa).
The C1 esterase inhibitor (C1-INH) enzyme plays a role in the classical pathway of the complement cascade, which is a component of the immune system response that acts to protect the human body from a variety of foreign substances. [5]
As a result, C1-inhibitor levels of less than 50% of the standard lead to increased vascular permeability, characteristic of angioedema. [12] Cinryze, a human plasma derived C1-esterase inhibitor, has been approved for use in 2008 for the prevention of hereditary angioedema attacks. [14] [15]
Santarus and Pharming Announce Poster Presentation of Pivotal Clinical Data for RUCONEST (Recombinant Human C1 Esterase Inhibitor) SAN DIEGO & LEIDEN, Netherlands--(BUSINESS WIRE)-- Santarus, Inc ...
Normal C1 inhibitor level hereditary angioedema is thought to involve various mutations that increased bradykinin activity and cause a decreased threshold for activation of the plasma contact system thus leading to the symptoms of angioedema. [7] Hereditary angioedema with normal C1-inhibitor is a genetically heterogeneous disorder.
A C1-INH concentrate can be used for angio-oedema (C1-INH deficiency). [2] [3] Pneumococcus and Haemophilus infections can be prevented via immunization. [2] Epsilon-aminocaproic acid could be used to treat hereditary C1-INH deficiency, though the possible side effect of intravascular thrombosis should be weighed. [7]
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